SPECIFICITY OF THE HUMAN INTESTINAL DISACCHARIDASES AND IMPLICATIONS FOR HEREDITARY DISACCHARIDE INTOLERANCE*

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Abstract
By heat inactivation of preparations from human intestinal mucosa the existence of at least 6 different disaccharidases has been demonstrated: isomaltase (= maltase I a), invertase (= maltase I b), maltase II, maltase III, trehalase and lactase (= cellobiase). Hereditary disaccharide intolerance is a group of inborn errors of metabolism when one or another of the intestinal disaccharidases is missing. From the specificity of the human disaccharidases it can be predicted that intolerance for isomaltose, sucrose, trehalose, lactose and cellobiose can occur as the result of isolated enzyme defects. Intolerance for maltose, in contrast, requires the simultaneous absence of several different enzymes. As far as hitherto recognized cases of disaccharide intolerance have been investigated, they accord with this theory.